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Biomedical subjects

Chuen Hsueh

Publications and source records attributed to Chuen Hsueh.

At least 55 records · Page 3Linked to original sources

Ductal adenocarcinoma of the pancreas with huge cystic degeneration: a lesion to be distinguished from pseudocyst and mucinous cystadenocarcinoma.

Cystic neoplasms of the pancreas are rare and often mistaken for pseudocyst by imaging studies and macroscopic examination. We describe an unusual tumor of the pancreas composed of a mural nodule of anaplastic carcinoma arising from a huge ductal adenocarcinoma undergoing cystic degeneration. The cyst measured 27 x 13 x 4 cm. Light microscopy showed that the cyst was partly lined by a single layer of cuboidal to columnar tumor cells with focal mucin production and was surrounded by hyalinized connective tissue. Most lining epithelial cells were absent owing to extensive degenerative process. Immunohistochemical studies showed positive staining of cytokeratin and vimentin for pleomorphic giant tumor cells, which were negative for leukocyte common antigen (CD45), KP-1 (CD68), epithelial membrane antigen (EMA), and carcinoembryonic antigen (CEA). The ductal adenocarcinoma stained strongly positive for cytokeratin and EMA, and negative for vimentin, CD45, CD68, and CEA. The clinical course of the current case was extremely poor and the prognosis resembled that of an anaplastic carcinoma. Therefore, we like to emphasize the importance of complete excision and extensive sampling of any cystic neoplasms in the pancreas including those with large cystic component to avoid missing the malignant elements.

Carcinoma↗

Congenital mixed malignant germ cell tumor involving cerebrum and orbit.

Congenital intracranial tumors are rare and only account for 0.5 to 1.5% of all pediatric brain tumors. Teratoma is the most frequently encountered intracranial tumor at birth. Cephalomegaly and hydrocephalus are the usual clinical presentations. Advances in imaging techniques have improved diagnostic accuracy in congenital brain tumors. But the prognosis of a massive intracranial teratoma is always dismal. We report a congenital mixed malignant germ cell tumor in a male fetus at 27 weeks of gestation, with massive involvement of cerebrum and orbit. According to histological and immunohistochemical studies, the tumor was made up of predominantly immature teratoma combined with a yolk sac tumor. To our knowledge, such a combination has not been well documented in the literature.

Adult↗

Adrenal incidentalomas in Taiwan: high prevalence and malignancy rate.

BACKGROUND: The endocrine adrenal gland has 3 zones comprised of the cortex and medulla. The character of multi-hormonal expression results in multiple clinical manifestations. To define the clinical characteristics of adrenal tumors in Taiwan, we reviewed 336 pathologically proven adrenal tumors at Chang Gung Memorial Hospital in Linkou. METHODS: We retrospectively analyzed 258 pathologically proven adrenal tumors with sufficient data treated from 1983 to 2000. Among them, 174 (67%) were functional and 84 (33%) were nonfunctional. The diagnosis was based on clinical features, hormonal status, imaging studies, and pathology. RESULTS: Of the 258 patients, 161 were women (mean age, 38.5 +/- 15.4; range, 1-75 years) and 97 men (mean age, 41.6 +/- 17.8; range, 1-81 years). The prevalence of cortical tumors was much higher in females than in males. Fifty-two percent of patients with an adrenal tumor were diagnosed at an age between 30 and 50 years. Incidentalomas (N = 84) accounted for about 1/3 of total tumor cases. Of the 46 cases proven to be malignant, 40 (87%) were found incidentally. All malignant tumors had a diameter of greater than 3 cm. Postoperative adrenal insufficiency was present in 18% of cases, and overall mortality in this study was 0.4%. CONCLUSIONS: In our series, adrenal incidentalomas corresponded to about 1/3 of adrenal tumors and accounted for 87% of malignancies. It is mandatory to increase medical attention for incidentally found adrenal masses in Taiwan. Those incidentalomas with a diameter of more than 3 cm should undergo a pathological examination.

Adolescent↗

Pathological analysis of congenital cervical cysts in children: 20 years of experience at Chang Gung Memorial Hospital.

BACKGROUND: Congenital cervical cysts are frequently encountered in pediatric populations, and constitute one of the most intriguing areas of pediatric pathology. This report analyzes cervical cysts in Taiwanese children diagnosed at Chang Gung Memorial Hospital (CGMH) over the past 20 years. The pathologic and clinical findings are reviewed. METHODS: Files on 331 patients under the age of 18 years, with a diagnosis of congenital cervical cyst at CGMH from January 1, 1983 to June 30, 2002, were retrieved from the Department of Pathology. There were 204 boys and 127 girls. We reviewed the histology of all cases and correlated it with clinical information in the medical records. RESULTS: Thyroglossal duct cysts, the most common congenital neck cyst, accounted for 54.68% of all cases, followed by cystic hygromas (25.08%), branchial cleft cysts (16.31%), bronchogenic cysts (0.91%), and thymic cysts (0.30%). Nine cases (2.72%) remained unclassified. CONCLUSIONS: This is the largest series regarding pediatric cervical cysts in the literature to date. Thyroglossal duct cysts were the most common congenital cervical cyst encountered. Our experience indicates that each type of cyst has its unique location in the neck and is highly associated with its embryonic origin. Complete and precise clinical information is a prerequisite in order for pathologists to make accurate diagnoses of congenital cervical cysts.

Branchioma↗

Macrocystic serous cystadenoma of the pancreas in a young patient resembling a pseudocyst: case report and literature review.

Macrocystic serous cystadenoma is an unusual and essentially benign pancreatic tumor. Ages of reported cases are usually 60 years and over, with a mean age of 54 years. Herein, we report on a 26-year-old man who presented with upper abdominal pain. A cystic lesion in the mid-portion of the pancreas was revealed by abdominal computed tomography, and a pseudocyst was suspected. A distal pancreatectomy was performed with a splenectomy due to intractable abdominal pain and being unable to rule out to be a mucinous cystic neoplasm, which has a malignant potential. The histopathological diagnosis was macrocystic serous cystadenoma of the pancreas. To our knowledge, this patient is the youngest person to present with such tumor. Clinical and pathologic features including complete immunohistochemical studies are presented, and we review the relevant literature.

Adult↗

The 1998 enterovirus 71 outbreak in Taiwan: pathogenesis and management.

The most recently discovered enterovirus, enterovirus 71 (EV71), is neurotropic and may cause severe disease and sudden death in children. In 1998, a large outbreak of enterovirus infection occurred in Taiwan that resulted in 405 severe cases in children and 78 deaths. Of the 78 children who died, 71 (91%) were <5 years old. EV71 was the primary agent in fatal cases of infection. Most of these patients died within 1-2 days of admission to the hospital. We hypothesize that EV71 directly attacks the central nervous system and causes neurogenic pulmonary edema and cardiac decompensation through the mechanism of sympathetic hyperactivity and inflammatory responses. Early recognition of risk factors and intensive care are crucial to successful treatment of this fulminant infection. After poliovirus is eradicated, EV71 will become the most important enterovirus that affects children, and development of a vaccine may be the only effective measure against it.

Convalescence↗

Malignant rhabdoid tumour of the kidney occurring simultaneously with a brain tumour: a report of two cases and review of the literature.

UNLABELLED: Malignant rhabdoid tumour of the kidney (MRTK), an uncommon aggressive neoplasm of children, is now recognised as a separate entity from Wilms' tumour with distinct clinical and pathological features. MRTK is unique in its significant association with primary brain tumours or brain metastases. We report two cases, aged 2 and 6 months, of MRTK occurring concurrently with a brain tumour. Radical nephrectomy and ventriculo-peritoneal shunting were performed. Both patients expired 2 and 6 months later despite receiving aggressive post-operative chemotherapy and radiotherapy. CONCLUSION: malignant rhabdoid tumour of the kidney is an uncommon neoplasm of early childhood with a poor prognosis. Due to its significant association with brain tumours or early brain metastases, concurrent brain computer tomographic examination is essential for all patients with this disease.

Brain Neoplasms↗

Anaerobic thyroid abscess from a thyroid cyst after fine-needle aspiration.

BACKGROUND: Anaerobic abscess formation within a thyroid cyst is rare but still possible, although aerobic thyroid abscess formation in the thyroid gland after fine-needle aspiration (FNA) has been observed in immunocompromised patients. METHODS: This study describes the clinical manifestations, thyroid echography, cytologic finding, culture outcome, and treatment course of an anaerobic abscess formation within a thyroid cyst after FNA in a healthy subject. RESULTS: A 53-year-old male subject had rapid enlargement of a left thyroid cyst develop after second FNA. Frank pus was obtained through third FNA. The culture outcome was Propionibacterium acnes, which was rich in saliva and one of the pathogens causing periodontitis and gingivitis. After adequate antimicrobial therapy, the abscess gradually diminished. CONCLUSIONS: This article reported, for the first time, on the formation of an anaerobic thyroid abscess after FNA in a healthy subject. We recommended careful aseptic procedure and adequate isolation processes, such as wearing a mask to avoid an unfavorable outcome as a result of a bacterial infection.

Abscess↗

Significance of postoperative serum thyroglobulin levels in patients with papillary and follicular thyroid carcinomas.

BACKGROUND AND OBJECTIVES: Although there are many factors that affect postoperative serum levels of thyroglobulin (Tg), such levels have been previously used to detect recurrence of papillary and follicular thyroid carcinomas. This study was conducted to elucidate the significance of postoperative levels of Tg in patients with clinical presentations of papillary thyroid carcinoma, follicular thyroid carcinomas, or both. METHODS: To collect data pertaining to patients with thyroid cancer who were treated in Chang Gung Medical Center in Linkou, Taiwan, records relating to a total of 847 patients with pathologically verified papillary or follicular thyroid cancer, all of whom received total thyroidectomy and thyroid remnant ablation with radioactive iodide ((131)I), were studied. To evaluate the clinical significance of postoperative levels of Tg, the patients were categorized into three groups based on postoperative Tg level. Group A was classified as those demonstrating a 1-month postoperative Tg levels less than 1 ng/ml. Group B patients were classified as those displaying a 1-month postoperative Tg levels greater than or equal to 1 ng/ml, but less than 10 ng/ml. Group C patients were classified as those exhibiting a 1-month postoperative Tg levels great than or equal to 10 ng/ml. RESULTS: Of the patients in group A, none presented with distant metastases at the time of diagnosis or during the follow-up period. In group B, 15 patients (3.5%) died of thyroid cancer. In this group, tumor size was an important factor in cancer-related mortality, diagnostic clinical class, and follow-up status. Of the 491 patients in group C, 49 (10.0%) patients died of thyroid cancer. Among the patients in group C, age, histopathologic type, stage of diagnosis, and follow-up Tg values were important factors. Among groups A, B, and C, there were 161 (95.8%), 253 (76.4%), and 129 (37.1%) patients, respectively, with disease-free status at the end of 1998. CONCLUSIONS: Postoperative serum Tg levels can be used as a prognostic indicator in patients with papillary and follicular thyroid cancer. For patients with Tg levels greater than or equal to 10 ng/ml, Tg levels are a useful marker to predict prognosis.

Adult↗

Adriamycin-induced cardiomyocyte and endothelial cell apoptosis: in vitro and in vivo studies.

Adriamycin is a potent, broad-spectrum chemotherapeutic agent effective against solid tumors and malignant hematological disease. The major limiting factor for adriamycin is its cardiotoxicity. Thus, the objective of this study was to investigate the role of cardiomyocyte and endothelial cell apoptosis in adriamycin-induced cardiomyopathy, in vivo and in vitro. For in vivo study, intraperitoneal injections of adriamycin were administered to nine adult male Wistar rats and normal saline to six rats as control. Eight of the nine rats in the adriamycin group, but none in the control group, developed marked ascites and DNA ladders in agarose gel electrophoresis of genomic DNA extracted from the rat hearts (P<0.001). The ratio of apoptotic nuclei in the cardiomyocytes was significantly higher for the adriamycin-treated rats (162+/-149/10(6) cells) than for the controls (4.2+/-1.3/10(6) cells; P<0.01) by TUNEL assay. Increased endothelial cell apoptosis was detected in the small coronary vessels of the myocardium of the adriamycin-treated rats. Increased immuno-reactive Caspase-3 expression was also noted for both cardiomyocytes and endothelial cells of adriamycin-treated rats. In vitro adriamycin treatment for cultured neonatal rat cardiomyocytes and human umbilical vein endothelial cells, respectively, showed a dose-related increase in apoptosis as determined by flowcytometry, DNA ladder analysis, TUNEL assay and/or electron-microscope examination. A dose-related increase in the expression of Fas antigen, Bax and Caspase-3, as well as a decrease in the expression of Bcl-2, were determined for the adriamycin-treated cardiomyocytes using Northern blot analysis, reverse transcriptase polymerase chain reaction (RT-PCR) and ribonuclease protection assay. RT-PCR also revealed increased Fas antigen expression, decreased Bcl-2 expression, and no change in Bax expression for the adriamycin-treated human umbilical vein cells. Further, pretreatment with broad caspase inhibitor, but not neutralizing FasL antibody, resulted in inhibition of adriamycin-induced endothelial cell apoptosis. In conclusion, these results indicate that both adriamycin-induced cardiomyocyte and endothelial cell death can occur via apoptosis which is dose-related, and can occur both in vitro and in vivo with changes in the expression of the apoptosis-related genes. Adriamycin-induced endothelial cell apoptosis is mediated by caspase activation but is Fas/FasL signal pathway independent. Our data provides evidence that both cardiomyocyte and endothelial cell apoptosis may play an important role in adriamycin-induced cardiomyopathy.

Amino Acid Chloromethyl Ketones↗

Infrequent p53 gene mutations and lack of p53 protein expression in clear cell sarcoma of the kidney: immunohistochemical study and mutation analysis of p53 in renal tumors of unfavorable prognosis.

A high prevalence of p53 gene mutation and protein expression has been found in the anaplastic variant of Wilms' tumor (WT), known to be associated with poor outcome. However, there are very few studies of p53 alterations in the other two rare and highly malignant renal tumors in childhood, in other words, clear cell sarcoma of the kidney (CCSK) and malignant rhabdoid tumor of the kidney (MRTK). Overexpression of p53 protein has been detected in eight CCSKs in one study, and in two in another, yet no molecular correlation with p53 gene mutations has been carried out. Our study is the first molecular analysis concerning p53 in CCSK. We investigated eight cases of CCSK and one case of MRTK for p53 protein expression by immunohistochemical staining. All were analyzed for p53 mutations in the region of exons 4 to 8 by polymerase chain reaction-single-strand conformational polymorphism (PCR-SSCP) method and DNA sequencing analysis. By histological study, no CCSK showed anaplastic features. None expressed p53 protein, but two harbored p53 mutations. One was in exon 5, with a base pair insertion between codons 162 to 163 causing frameshift alteration in amino acid. Another was a silent CTC-->CTT transversion in codon 289 of exon 8. The case of MRTK did not show any alterations of p53 protein or gene. Our result indicates that p53 alterations are infrequent in CCSK and do not seem to be primary genetic events in the pathogenesis of CCSK.

Adolescent↗

Hepatoblastoma in a child of extremely low birth weight.

Hepatoblastoma is a rare childhood embryonal malignancy. Recent reports suggested a linkage between premature birth and later development of hepatoblastoma. In August 2000, we encountered a 24-month-old boy with extremely low birth weight who developed hepatoblastoma with an unfavorable histology. His birth weight was only 584 g. Radiological evaluation of the liver revealed a hypoattenuated tumor occupying the left lobe. His initial serum alpha-fetoprotein level was 4032 ng/mL. The patient underwent successful left hepatectomy. The histological examination showed that the tumor, weighing 210 g, was a hepatoblastoma of epithelial type in two different patterns. One was composed purely of fetal type of tumor cells, whereas another was made up of embryonal type of cells in a macrotrabecular pattern. Four courses of adjuvant chemotherapy were given after surgery and were well tolerated. The patient remained in clinical remission 18 months after diagnosis.

Child, Preschool↗

Cutaneous lymphocytic vasculitis as the presenting feature of acute lymphoblastic leukemia.

The authors describe a patient with precursor B-cell acute lymphoblastic leukemia who presented with a 3-week history of indurated or ulcerative, purpuric lesions distributed mainly on her legs. A skin biopsy demonstrated a T-cell-mediated lymphocytic vasculitis. After the patient started chemotherapy, the skin lesions abated but she became febrile and a blood culture revealed cryptococci. The cryptococcal infection was successfully treated first with amphotericin B and later with fluconazole. The relationship between lymphocytic vasculitis and acute lymphoblastic leukemia may be an example of paraneoplastic association because both conditions seem to have appeared at about the same time, and both followed a parallel course. Lymphocytic vasculitis may also reflect a new manifestation of host-leukemia interaction.

Anti-Bacterial Agents↗

Spontaneous intracranial hemorrhage caused by pheochromocytoma in a child.

We report a 6-year-old boy who presented with left occipital spontaneous lobar hematoma and hypertension. Cerebral angiography failed to demonstrate a vascular malformation. Subsequently, a left adrenal tumor was found that proved to be a pheochromocytoma. This is the first report in the literature of a lobar hematoma in a child caused by a concomitant pheochromocytoma. In children with hypertension and resulting intracerebral hematoma, a pheochromocytoma should be considered in the differential diagnosis.

Adrenal Gland Neoplasms↗

Undifferentiated sarcoma of the liver.

Undifferentiated (embryonal) sarcoma of the liver (USL) is a rare primary hepatic malignancy principally affecting patients of pediatric age. It is believed to be a primitive mesenchymal neoplasm, which usually behaves in a highly malignant fashion. The median survival has been less than a year. The only chance for cure appears to be radical excision of the tumor. However, some patients may develop recurrent disease despite complete surgical resection of the tumor. Recently, long-term disease-free survival has been achieved in cases which underwent aggressive multimodal treatment. Herein we report on a 12-year-old girl who underwent surgical excision of USL. Histologically, it was composed of pleomorphic stellate or spindle-shaped cells in a myxoid stroma. Characteristic periodic acid Schiff-positive, diastase-resistant intracytoplasmic hyaline globules were seen. Immunohistochemically, the tumor cells were positive for vimentin, alpha1-antitrypsin, and alpha1-antichymotrypsin. In addition, p53 protein was expressed in 40% of tumor cells, and Ki-67 was demonstrated in 45% of tumor cells. Postoperative chemotherapy was recommended, but was refused by the patient's family. A recurrent liver mass was found 171 days after the operation. Chemotherapy was refused once again by the patient's family, and she was lost to follow-up for 5 months. The patient was brought to the outpatient clinic again because of abdominal fullness and back pain. The recurrent tumor was 20 cm in size with compression of the inferior vena cava. She was admitted and received chemotherapy with vincristine, ifosfamide, and cisplatin.

Child↗

Unusual cardiac papillary fibroelastoma in the right ventricular outflow tract.

Cardiac papillary fibroelastoma (CPF) is the second most common benign neoplasm of the heart. This study describes the case of an 81-year-old man who was admitted to the hospital for severe vertigo and in whom a tumor at the right ventricular outflow tract (RVOT) was identified incidentally during echocardiography. The CPF was excised smoothly following the confirmation of its position by computed tomography. The comprehensive pathologic findings of CPF were reviewed. Detailed immunohistochemical analyses of CD34 and factor VIII-related antigen were performed on the covering endocardial cells. The unique chondroid metaplasia of fibrous tissue in this CPF has never been reported. This work is the first to present an unusual CPF at the RVOT with reactive process of fibrous connective tissue.

Aged↗

Primary intraspinal mesenchymal chondrosarcoma: report of one case.

We report a case of primary intraspinal mesenchymal chondrosarcoma in an 11-year-old boy. Contrast-enhanced MRI revealed a hyperintense, sausage-shaped mass extending from C7 to T2. The lesion located anterolaterally on the left side, pushing the spinal cord to the right and posterior. Microscopic examination and immunohistochemical studies confirmed the diagnosis of mesenchymal chondrosarcoma. Follow-up studies have shown no tumor recurrence for 2 years. This tumor is rare but should be considered for differential diagnosis in spinal tumors.

Child↗